| Autor: |
Cohen-Cymberknoh M; Pediatric Pulmonary Unit, Cystic Fibrosis Center and Sleep Unit, Hadassah Medical Center, Jerusalem 9112001, Israel.; Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 9112102, Israel., Lehavi M; Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 9112102, Israel.; Department of Military Medicine and 'Tzameret', Faculty of Medicine, Hebrew University of Jerusalem, and Medical Corps, Israel Defense Forces, Jerusalem 9190501, Israel., Gileles-Hillel A; Pediatric Pulmonary Unit, Cystic Fibrosis Center and Sleep Unit, Hadassah Medical Center, Jerusalem 9112001, Israel.; Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 9112102, Israel., Atia O; Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 9112102, Israel.; Department of Pediatrics, Shaare Zedek Medical Center, Jerusalem 9103102, Israel., Breuer O; Pediatric Pulmonary Unit, Cystic Fibrosis Center and Sleep Unit, Hadassah Medical Center, Jerusalem 9112001, Israel.; Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 9112102, Israel., Reiter J; Pediatric Pulmonary Unit, Cystic Fibrosis Center and Sleep Unit, Hadassah Medical Center, Jerusalem 9112001, Israel.; Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 9112102, Israel. |
| Abstrakt: |
Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are associated with sleep disturbances affecting quality of life (QOL) in both children and adults. However, little is known about the progression of these complaints over time, and the effect of CFTR modulator (CFTRm) therapies. Participants completed sleep quality (SDSC, PSQI) and quality of life questionnaires (PedQL, QOL-BE) as well as the Epworth sleepiness scale (ESS) at baseline and after 4 years. Medical records were reviewed for clinical data and correlations were sought between sleep, QOL, and clinical parameters. A total of 67 patients (33 pediatric), 37 pancreatic insufficient CF (CF-PI), 15 pancreatic sufficient CF (CF-PS), and 15 PCD patients, completed the study. In adults, global sleep quality decreased from 85.8% (76.2-90.5) to 80.9% (71.4-85.7); ( p = 0.009). Analysis by disease cohort showed a significant deterioration only in the CF-PS group. In adults off CFTRm, sleep quality decreased from 85.7% (78.6-88.2) to 80.9% (71.4-87.3); ( p = 0.021) and from 85.8% (76.2-92.9) to 76.2% (71.4-85.8); ( p = 0.078) in people on CFTRm. Changes in sleep quality and changes in QOL over time were strongly associated with each other. In conclusion sleep quality deteriorates over time, correlates with QOL, and is driven primarily by adults and CF-PS patients. CFTRm has a possible effect on sleep initiation; however, results are mixed, and further long-term studies are required. |
